A brain stem glioma is a tumor occurring in the brain stem. It may be benign or malignant. The condition most commonly occurs in children. The two forms of pediatric brain stem glioma are diffuse intrinsic pontine glioma and focal or low-grade glioma. The majority of pediatric brain stem gliomas arise within the pons; the rest occur in the medulla, midbrain, or cervicomedullary junction. Typically, pontine tumors are high-grade and locally infiltrative tumors with a poor prognosis. Patients with focal or low-grade brain stem gliomas have a much better prognosis. Patients may present with symptoms of hydrocephalus such as headache, nausea, vomiting, and papilledema. Other common symptoms include unsteady gait, visual and hearing impairment, dysphagia, dysarthria, hemiparesis, and drowsiness.
Patients with certain genetic disorders such as neurofibromatosis type 1 have an increased risk of developing a brain stem glioma. Clinical presentations may vary depending on patient's age and specific tumor location. Prognosis varies depending on the tumor type, grade, and location. Treatment should be overseen by a team of expert health care providers specializing in brain tumors in that age group. Standard therapeutic approaches consist of radiation therapy, chemotherapy, and cerebrospinal fluid diversion. Surgery is often not possible due to the high-risk location of these tumors.